Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.
Angiography ; Catheterization ; Catheters ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Drainage ; Estrogens, Conjugated (USP) ; Humans ; Hypertension ; Lung ; Magnetic Resonance Imaging ; Perfusion ; Pneumonectomy ; Pulmonary Edema ; Pulmonary Veins* ; Radiography, Thoracic ; Veins

PURPOSE: The purpose of this study was to analyze the MR findings of childhood adrenoleukodystrophy regarding the extent of white matter degeneration and the pattern of enhancement, and to correlate these findings with clinical manifestations. MATERIALS AND METHODS: Six male patients (5-13 years old;mean age, 8.7 years) of biochemically confirmed adrenoleukodystrophy were included in this study. We evaluated the extent of white matter degeneration which was shown as high signal intensity on T2-weighted image, and the pattern of enhancement. Also we correlated the MR findings with clinical manifestations of symptoms and the results of evoked potential studies. RESULTS:The most common sites of predominant involvement were occipital deep white matter, optic radiation, and splenium of corpus callosum (n = 4, 66.7%). The other sites of predominant involvement were frontal deep white matter (n = 1, 16.7%) and pyramidal tract with cerebellum (n = 1, 16.7%). After contrast enhancement, there was peripheral rim enhancement surrounding the most severely affected areas in five cases (83. 3%). These findings were consistent with clinical symptoms in five cases (83.3%), but the results of evoked potential studies were consistent with clinical symptoms in only two cases (33.3%). CONCLUSION:In childhood adrenoleukodystrophy, MRI can clearly demonstrate specific tracts of involvement and these findings were well correlated with clinical manifestations.
Adrenoleukodystrophy* ; Cerebellum ; Corpus Callosum ; Evoked Potentials ; Humans ; Magnetic Resonance Imaging* ; Male ; Pyramidal Tracts

We report two patients with Hallervorden-Spatz disease, who were diagnosed by same MR findings of marked low signal intensity in the globus and substantia nigra. They presented with ataxic and spastic gait, intention tremor, delayed mental development, and dysarthria. They were 7 year-old male and 8 yea r-old female siblings, who were healthy until 3 years of age when they suffered from progressive symptoms. T2-weighted images showed marked low signal intensity in the globus pallidus and substantia nigra indicating an increased irondeposition, and it might suggest Hallervorden-Spatz disease.
Child ; Dysarthria ; Female ; Gait Disorders, Neurologic ; Globus Pallidus ; Humans ; Male ; Pantothenate Kinase-Associated Neurodegeneration* ; Siblings* ; Substantia Nigra ; Tremor

PURPOSE: Chronic granulomatous disease(CGD) is a group of genetic disorders characterized by recurrent pyogenic infections of the respiratory tract, skin,and soft tissue. The aim of this study is to describe the radiological findings of CGD. MATERIALS AND METHODS: We retrospectively analyzed radiological findings of 11 patients of CGD, which were diagnosed by nitroblue tetrazolium test. We analyzed the pattern of pneumonia on chest radiograph in all infants. Three cases of chest CT and one case of digital subtraction angiography were performed. According to infant's symptom, abdominal ultrasonography(n=8), abdominal CT(n=5), simple bone radiography(n=2), and brain CT(n=I) were performed. RESULTS: Repeated infiltration(100%), mass-like consolidation(73%), hilar or mediastinal lymph node enlargement(64%), scattered nodules(55%), cavity formation(27%), and pleural effusion(27%) were found on the chest radiographs(n=11) and CT(n=3). On the abdominal imagings(n=8), there were hepatosplenomegaly(n=6), calcifications in the liver(n=2) and kidney(n=1), hepatic granuloma(n=1) and renal abscess(n=1). Two patients had osteomyelitis. There were findings of meningitis on brain CT(n=1). CONCLUSION: We believe that the combinations of these radiological findings may suggest the diagnosis of CGD in patients with history of reccurent infection.
Angiography, Digital Subtraction ; Brain ; Diagnosis ; Granulomatous Disease, Chronic* ; Humans ; Infant ; Lymph Nodes ; Meningitis ; Nitroblue Tetrazolium ; Osteomyelitis ; Pneumonia ; Radiography, Thoracic ; Respiratory System ; Retrospective Studies ; Thorax ; Tomography, X-Ray Computed

BACKGROUND: HLA-B40 is the most frequently identified HLA-B type in Koreans. Also HLA-B60 and B61 are the serologic split antigens of HLA-B40. But because of the lack of mono-specific alloantisera, cross reactivity of sera used as typing reagents, and poor antigenicity of some specific cells such as cord blood lymphocytes, discrimination between HLA-B60 and B61 has been often problematic in laboratories. In this study, authors evaluated whether the PCR-SSP method can be useful for accurate assignments of HLA-B60 and B61 or not. METHODS: Twenty-nine lymphocytes samples which were suspected as heterozygotes or homozygotes of HLA-B60 or B61 and three samples typed as HLA-B40 are selected from stored cord blood and organ transplantation donors. HLA types of these samples were defined by serologic method using a commercial typing kit. PCR that amplified exons 2 and 3 of the HLA-B gene using sequence specific primer pairs exactly matched to HLA-B60 or B61 allele making up a serological specificity was done. RESULTS: A clear discrimination between B60 and B61 was possible in all samples including 9 serologically ambiguous samples. Discrepancy between serologic typing and molecular typing was seen in three cases identified serologically as B40 positive but inable to define a split. Among three samples, two were identified as HLA-B61 and one was identified as HLA-B60. CONCLUSIONS: Molecular typing was useful in discriminating between HLA-B60 and B61. The PCR-SSP method for HLA-B60 and B61 including other cross-reactive HLA types will be helpful as a supplemental method of the serologic typing.
Alleles ; Discrimination (Psychology)* ; Exons ; Fetal Blood ; Heterozygote ; HLA-B Antigens ; HLA-B40 Antigen ; Homozygote ; Humans ; Indicators and Reagents ; Lymphocytes ; Molecular Typing ; Organ Transplantation ; Polymerase Chain Reaction* ; Sensitivity and Specificity ; Tissue Donors ; Transplants

Respiratory syncytial virus is the most common cause of lower respiratory infection (bronchiolitis and pneumonia) of infancy and early childhood. We analyzed clinical and radiological features of 76 patients with lower respiratory infections by respiratory syncytial virus, which were diagnosed by indirect immunofluorescent test or culture of nasal aspirate in Hep-2 cell monolayer, during the period of January-December, 1991. There were peaks of incidences in March-May and November-December, accounting for 87% of eases. Sixty-two cases (82%) were under 1 year of age. Fifty cases(66%) had underlying diseases. Major radiographical findings were overaeration (83%), parahilar peribronchial infiltrates(67%), segmental or subsegmental atelectasis(32%), and segmental or lobar consolidation(16%). In 15 cases(20%), overaeration was the only radiological findings. There was no evidence of pleural effusion or hilar lymph node enlargement in all cases. By considerig clinical features(symptoms, age. Underlying diseases, epidemic seasons) in addition to the radiological findings, radiologists would be familiar with lower respiratory infection by respiratory syncytial virus. Air space consolidation, which is generally though to represent bacterial pneumonia, is also observed not infrequently in respiratory syncytial virus infections.
Child* ; Humans ; Incidence ; Infant* ; Lymph Nodes ; Pleural Effusion ; Pneumonia, Bacterial ; Respiratory Syncytial Virus Infections ; Respiratory Syncytial Viruses* ; Respiratory Tract Infections

PURPOSE: Pediatric sedation is an important factor for obtaining the images of good quality. We performed this study to analyze the efficacy of our sedation protocol using chloral hydrate. MATERIALS AND METHODS: We collected prospectively 151 sedation records of children(1 day-15 years old), who were sedated with chloral hydrate for MR(n=112) or CT(n=39) studies. We initially administered 50mg/Kg orally(n=94) or rectally(n=57) 30 minutes before the scheduled examinations, and then administered additionali dose (second dose :25-35mg/Kg, third dose:10-15mg/Kg) to patients whom initial dose failed to sedate. RESULTS: Satisfactory sedation was achieved by initial administration in 109 patients(72%) without si difference between oral(per oral: P.O.) and rectal(per rectal: P.R.) administration. Second dose was required in 28% and third dose in 5%. MR and CT examinations required second dose in 36(32%) and 6 patients(15%), respectively. P.O. -patients vomited in 5%. P.R. -patients defecated in 22% after initial administration. There were no other serious complications. Time interval from the drug administration to the start of examinations was 33 minutes in initial-dose-group and 64 minutes in additional-dose-group. Two patients could not complete MR examination due to early arousal. Prolonged sedation, requiring more than 30 minutes for alertness after MR and CT examinations, was encountered in twenty(18%) and two patients(5%), respectively. CONCLUSION: Our protocol using chloral hydrate(P.O. or P.R.) is thought to be an effective and safe method for pediatric sedation for MR or CT imaging.
Arousal ; Child* ; Chloral Hydrate* ; Humans ; Prospective Studies

MR imaging of the brain in 34 patients were reviewed to characterize the MR findings of brain damage due to perinatal hypoxic insult All the patients had a history of perinatal hypoxia and showed abnormal brain MR findings. Out of 34, eight infants were born premature and twenty-six were born at term or post-term. MR findings were analysed for the extent and location of abnormalities of the white matter, cortical abnormality. Corpus callosum atrophy, and abnormal progression of myelination. The major abnormalities were abnormal signal lesions or atrophy of the cerebral white matter and gyral atrophy of the cerebral cortex. The distribution of white matter lesions well correlated with maturity of the brain at the time of hypoxic insult. Periventricular and deep white matter lesions predominated in the premature-born patients. Corpus callosum atrophy was frequently seen. Reflecting the location of white matter injury. Delay in myelination was present in 55%. MR is a very useful diagnostic imaging modality and guide for the prediction of prognosis by accurate depiction of the location and extent of brain damage due to perinatal hypoxic insult.
Anoxia* ; Atrophy ; Brain* ; Cerebral Cortex ; Corpus Callosum ; Diagnostic Imaging ; Humans ; Infant ; Magnetic Resonance Imaging ; Myelin Sheath ; Prognosis ; White Matter

PURPOSE: There have been repoty on radiological features of lower respiratory track infection in infants and children caused by RSV(respiratory syncytial virus) in KOREA. The aims of this study were to summarize radiological features of lower respiratory tract infection caused by common viral agents other than RSV and to find any specific radiological features which might provide clue to the etiologic diagnosis. MATERIALS AND METHODS: We retrospectively analyzed radiological features in 51 children with symptoms of lower respiratory tract infection and identification of viral agents(except RSV). They included parainfluenza (n=22), adenovirus(n=16), influenza A(n--11), influenza B(n=2) virus infections. The mean age of the patients was 23 months. RESULTS: Major radiological findings of viral lower respiratory tract infection were bilateral parahilar peribronchial infiltration(62%), bilateral overaeration(60%), atelectasis(59%)(segmental or subsegmental atelectasis(43%), Iobar atelectasis(16%)) and patchy or confluent consolidation(20%). Pleural effusion was seen in only one case and hilar adenopathy was not observed in any of them. In the cares of adeno virus, consolidation was seen in 5 cases(31%) including 3 cases919%) of extensive confluent consolidations and overaerations were less frequent findings(44%) than in other viruses. In 24 patients with radiological follow up for more than 1 week, consolidation improved most rapidly, while was persistent atelectasis. CONCLUSION: The major radiologic features in vital lower respiratory tract infection(except RSV) were overinflation, bilateral peribronchial infiltration and atelectasis. In adenoviral infection, confluent consolidations which are usually seen in bacterial pneumonia were more common findings than in other viral lower respiratory tract infections.
Child ; Diagnosis ; Follow-Up Studies ; Humans ; Infant* ; Influenza, Human ; Korea ; Paramyxoviridae Infections ; Pleural Effusion ; Pneumonia, Bacterial ; Pulmonary Atelectasis ; Respiratory System* ; Respiratory Tract Infections ; Retrospective Studies

Melanosis duodeni is an uncommon pathologic condition characterized by diffuse small black spots on the first and second portions of the duodenum. It occurs predominantly in elderly patients and is linked to chronic renal failure, use of antihypertensive drug and oral iron supplementation, and/or presence of gastric hemorrhage. We report a case of melanosis duodeni in a 60-year-old woman diagnosed with hypertension 20 years ago and chronic renal failure 6 years ago. She has taken antihypertensive drugs for many years. On endoscopy, speckled black pigmentation of duodenal mucosa was detected. Microscopically, this duodenal lesions showed numerous coarse pigments in macrophages confined within the lamina propria. By ultrastructural study, macrophages contained electron-dense granules admixed with lipofuscin-like material in residual bodies.
Aged ; Antihypertensive Agents ; Duodenum ; Endoscopy ; Female ; Hemorrhage ; Humans ; Hypertension ; Iron ; Kidney Failure, Chronic ; Macrophages ; Melanosis* ; Middle Aged ; Mucous Membrane ; Pigmentation